Tricuspid atresia in adults.

Congenital atresia of the tricuspid valve is still uncommon in adult patients. However, increasingly successful palliative surgery in children now has increased its incidence after age 15 years. This investigation updates the clinical features of this disease in adults in light of modern diagnostic and surgical techniques. The data on all 18 adults with tricuspid atresia having angiography after age 15 years at this institution since 1970 were reviewed. The patients' ages ranged up to 45 years; 12 had had previous palliative surgery. Left cineventriculography, particularly biplane, with the long axial view (60 degrees left anterior oblique with cranial angulation) is the most important diagnostic mode and reveals the ventricular and great vessel relations. According to standard classification, 11 patients had type I anatomy (normal great arterial relations), 4 type II (transposed great arteries) and 2 type III ("corrected transposition of the great arteries"). One patient with inverted ventricles could not be classified. Associated additional congenital defects were uncommon. On the basis of these data, a new anatomic classification of tricuspid atresia is given which encompasses all possible atrial-ventricular-great arterial combinations. Seven patients had further surgery after study, including two procedures of the Fontan type (right atrium to pulmonary arterial conduit). Follow-up data on all 18 patients revealed two deaths (one early after operation, one late after study without further surgery). The remaining 16 patients survive 2 to 120 months after study. Four patients had naturally balanced pulmonary and systemic circulations and have survived to ages 21 to 41 years without surgery. Prudent surgical decision based on accurate anatomic diagnosis and the need for optimal effective pulmonary blood flow may result in a relatively optimistic prognosis in adults with this disease.